Wegener granulomatosis: diagnosis and treatment

Wegener's granulomatosis

Wegener's granulomatosis- Systemic necrotic vasculitis of small veins and arteries with the formation of granulomas in vascular walls and surrounding tissues of the respiratory tract, kidneys, etc. bodies.

Wegener's granulomatosis is characterized by ulcerative necrotic rhinitis, changes in the larynx, paranasal sinuses, infiltration of pulmonary tissue with decay, fast-progressing glomerulonephritis, polymorphous skin rash, polyarthralgia. Diagnosis is based on the evaluation of clinical-laboratory, radiologic data, and biopsy results. With Wegener's granulomatosis, hormonal and cytostatic therapy is indicated; the prognosis of the development of the disease in generalized form is unfavorable.

The incidence of Wegener's granulomatosis is equally common among men and among women in the age group of about 40 years.

Classification of Wegener's granulomatosis

Given the prevalence of pathological changes, Wegener's granulomatosis can occur in a localized or generalized form.

In the development of Wegener's granulomatosis, four progressive stages are distinguished.

The first stage of granulomatous necrotic vasculitis, or rhinogenous granulomatosis, proceeds with a purulent-necrotic or ulcerative necrotic rhinosinusitis, laryngitis, nasopharyngitis, destructive changes in the osseous cartilaginous nasal septum and eye sockets. In the next stage (pulmonary), the parenchyma of the lungs is involved in the pathological process. At the third stage, generalized lesions develop with an interest in the lower respiratory tract, gastrointestinal tract, cardiovascular system, and kidneys. The last, terminal stage of Wegener's granulomatosis is characterized by pulmonary-cardiac or renal insufficiency, leading to the death of the patient in the coming year from the beginning of its development.

Symptoms of Wegener's granulomatosis

The general symptoms of Wegener's granulomatosis include fever, chills, increased sweating, weakness, weight loss, myalgia, arthralgia, arthritis.

The leading manifestation of the disease, which occurs in 90% of patients, is the defeat of the upper respiratory tract.

For the Wegener's granulomatosis clinic, a typically persistent course of rhinitis, a purulent-hemorrhagic discharge from nose, ulceration of the mucous membranes up to the perforation of the nasal septum, saddle deformation nose. Granulomatous necrotic changes also develop in the oral cavity, the larynx, the trachea, the auditory passageways, the paranasal sinuses.

Involvement of the kidneys is manifested by the development of glomerulonephritis with the phenomena of hematuria, proteinuria, impaired excretory function. The rapidly progressing course of glomerulonephritis with Wegener's granulomatosis leads to acute renal failure.

Changes from the skin manifest hemorrhagic rash followed by necrotic cells.

The defeat of the eyes includes the development of episcleritis (inflammation of the superficial sclera), education orbital granulomas, exophthalmos, ischemia of the optic nerve with impaired vision function up to its full loss. Wegener's granulomatosis affects the nervous system, coronary arteries, myocardium.

The localized form of Wegener's granulomatosis proceeds with the predominant lesion of the upper respiratory tract - coryza, obstruction of nasal breathing, epistaxis, formation of bloody crusts in nasal passages, hoarseness vote.

The generalized form is characterized by various systemic manifestations - fever, joint-muscle pains, hemorrhages, polymorphic rash, paroxysmal coughing with bloody-purulent sputum, abscessed pneumonia, cardiopulmonary and renal growth insufficiency.

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The complex of diagnostic procedures for Wegener's granulomatosis includes rheumatologist consultation, laboratory tests, diagnostic operations, X-ray studies.

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Clinical examination of blood reveals normochromic anemia, acceleration of ESR, thrombocytosis, general urine analysis - proteinuria, microhematuria.

In biochemical analysis of blood, an increase in γ-globulin, creatinine (in renal syndrome), urea, fibrin, seromucoid, haptoglobin is determined.

At a roentgenography of lungs in their tissue infiltrates, cavities of disintegration, a pleural exudate are defined. Bronchoscopy is performed with a biopsy of the mucosal upper respiratory tract. When studying biopsies, morphological signs of granulomatous necrotic vasculitis are revealed.

Treatment of Wegener's granulomatosis

With limited and generalized form of Wegener's granulomatosis, immunosuppressive therapy with cyclophosphamide in combination with prednisone is prescribed.

The rapidly progressing course of the alveolitis or glomerulonephritis serves as an indication for combined pulse therapy with high doses of methylprednisolone and cyclophosphamide.

In the course of the course of Wegener's granulomatosis, the dose of cyclophosphamide and prednisolone is gradually reduced to a remission stage; for a long (up to 2 years), appoint methotrexate.

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The generalization of the process for Wegener's granulomatosis (hemoptysis, glomerulonephritis, the presence of antibodies to neutrophilic leukocytes) requires extracorporal hemocorrection - cryoapheresis, plasmapheresis, cascade filtration of plasma, extracorporeal pharmacotherapy.

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In the treatment of recurrent granulomatosis Wegener is effective in the implementation of intravenous immunoglobulin therapy. For the purpose of prolonging remission, rituximab therapy may be provided.

Stenosing changes in the larynx with Wegener's granulomatosis require the imposition of a tracheostomy and a reanastomosis.

The course and progression of Wegener's granulomatosis can lead to destruction of facial bones, deafness due to persistent otitis, the development of necrotic granulomas in the lung tissue, hemoptysis, trophic and gangrenous changes of the foot, renal failure, secondary infections on the background of admission immunosuppressors. In the absence of therapy, the prognosis of Wegener's granulomatosis is unfavorable: 93% of patients die in the period from 5 months to 2 years.

The local form of Wegener's granulomatosis proceeds more benignly. Conducting immunosuppressive therapy improves the condition in 90 and persistent remission in 75% of patients.

The period of remission on average lasts about a year, after which 50% of patients have a new exacerbation. The progressing course of limited Wegener's granulomatosis, despite immunosuppressive therapy, is noted in 13% of cases.

We have not developed any measures for the prevention of Wegener's granulomatosis.

A source: http://www.krasotaimedicina.ru/diseases/rheumatology/wegener-granuloma

Wegener's granulomatosis: symptoms, diagnosis and treatment

Systemic vasculitis is a disease that is associated with inflammatory changes in the vascular wall. At the same time, capillaries, veins, venules, arterioles and arteries are affected.

Depending on which vessels have undergone inflammatory changes, possible damage to various organs and systems. Wegener's granulomatosis is a typical representative of the ANCA group-associated vasculitides.

It will be discussed in more detail.

The essence, causes and classification of pathology

On the etiology of vasculitis, the spores have not subsided. There are different versions of what can be associated with the appearance of the disease, for example:

• genetic mutations;
• infection with the Epstein-Barr virus;
• Persistence of such infectious agents as pneumocysts and Staphylococcus aureus;
• herpes simplex virus.

The key pathogenetic element is the synthesis of specific immunoglobulins. These antineutrophil cytoplasmic antibodies (that is, they are tropic to the cytoplasm of neutrophils) or ANCA. There are two varieties:

1. Myeloperkosidasa - in the detection of which the prognosis is much worse.
2. Proteinase-3 - this type of antibodies is detected with Wegener's vasculitis more often.

Proteinase-3 and less frequently occurring myeloperoxidase directly bind to neutrophils and monocytes circulating in the bloodstream.

On the membranes of the latter receptors are localized for binding to these immunoglobulins. When the antibody binds to the receptor, the cells become very active - they acquire high proteolytic activity.

This fact provides inflammation and necrotic destruction of the vascular wall.

These processes are accompanied by an increase in the concentration of proinflammatory cytokines, which in turn support the inflammatory process, involving new cellular and humoral elements.

Granulomatosis is classified according to the prevalence of vascular lesions. Classification is necessary to assess the prognosis and the possibility of treatment. Rheumatologists distinguish the following forms of the disease:

1. The local form.
2. Limited version of the disease.
3. A common granulomatosis.

For local lesions, it is typical to detect only the inflammatory process in the upper respiratory tract. This is rhinitis, sinusitis and much less often tracheitis and laryngitis. Hormonal and cytostatic treatment does not require this form of the disease.

Limited version of the disease involves the presence of lung damage. Clinically expressed symptoms usually do not. Only rarely cough, hemoptysis. More often pathological changes are observed on the roentgenogram of the lungs.

The common form is considered to be the heaviest.

Vascular kidneys are involved in the pathological process, which leads to the development of an isolated urinary syndrome or glomerulonephritis, sometimes with half-moon (fast-progressive variant). This option is considered an indication for aggressive cytostatic therapy courses.

This pathology occurs with the defeat of the capillary bed of the kidneys, skin, coronary arteries. But the most common variant of the debut is inflammatory necrotic damage to the upper respiratory tract.

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Dislocation of nasal mucosa, paranasal sinuses

The first manifestations of rhinitis may not be noticed because of the nonspecific manifestations. Rinorrhea, sneezing usually serves as an excuse for treating a respiratory viral infection. In the case of the described vasculitis, rhinitis becomes refractory to traditional symptomatic treatment.

Given that the process of inflammation is accompanied by the formation of granulomas and necrosis, the secretion from the nasal cavity will be bloody.

Patients report the appearance of an unpleasant smell during conversation and breathing.

A prolonged course of rhinitis leads to saddle-shaped deformation of the nose, which requires differential diagnosis with syphilitic damage.

The trachea and larynx are also the target of the granulomatous process in the context of a disease such as Wegener's granulomatosis.

The paranasal sinuses are involved in 65% of cases of granulomatosis. Characteristic appearance of such states as:

• sinusitis;
• etmoiditis (inflammation of the sinuses of the latticed bone);
• sphenoiditis;
• mastoiditis (damage to the cave of the temporal bone);
• fronts.

A dangerous complication of etmoiditis is the pseudotumor of the orbit, leading to loss of vision. It must be differentiated with tumors.

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Pathology of the lungs and kidneys in ANCA-vasculitis

This organ is involved in an inflammatory pathological process in about half of all cases of the disease. Wegener's granulomatosis is accompanied by damage to both the pulmonary tissue proper and the bronchial structures.

Coughing is very rare in vasculitis. It can appear with the development of dry pleurisy. It is often possible to record complaints of hemoptysis.

This symptom correlates with the serum myeloperoxidase content: the higher their titer, the more likely the appearance of hemophthosis.

It is important to note that this symptom almost never reaches the scale of bleeding.

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This disease with pulmonary lesions mostly occurs asymptomatically. Pathology can be seen only with the radiography of the lungs, confirming the definition of the titer of ANCA-antibodies.

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In the overwhelming majority of cases of ANCA-associated vascular vasculitis, the renal barrier is damaged with the development of glomerulonephritis.

Approximately 30-40% of patients with Wegener's vasculitis are registered with a nephrologist with a rapidly progressive form of glomerulonephritis.

The correlation of the frequency of development of this state with the concentration of proteinase-3 was revealed.

ANCA antibody

Features of renal damage in Wegener's disease are as follows:

1. A mild level of arterial hypertension (it is not of a malignant nature with high systolic pressure and high pulse pressure).
2. Absence of nephrotic syndrome.
3. Often occurs asymptomatically with isolated urinary syndrome in urinalysis.

Glomerulonephritis with a disease such as Wegener's granulomatosis is monitored. Diagnosis is based on ultrasound picture, functional indices and dynamics of antibody titer.

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What else is revealed in vasculitis?

Skin manifestations are associated with granulomatous inflammation of the skin vessels and subcutaneous fat. They are diverse, but more often there are such options:

• mesh livedo on the lower limbs or trunk;
• loose elements by type of urticaria;
• hemorrhagic rash;
• vascular phenomenon (syndrome) Reynaud.

The mesh is a liveno - the appearance of nodules in the subcutaneous fat, which are colored in a bluish or purple color. Their arrangement resembles a tree. Usually, the subject does not describe the subjective sensations.

Raynaud's syndrome manifests itself in the pallor of the fingertips, followed by their cyanosis and hyperemia. This is usually painful for the patient. This symptomatology is associated with a violation of the regulation of the lumen of blood vessels.

It is mainly located on the limbs, although it may appear in the trunk region. Half of patients may have eye damage. It is two-sided. A specific symptom is the appearance of the pseudotumor of the orbit.

Among other manifestations, one can follow all ophthalmic nosologies:

• uveitis;
• inflammation of the lacrimal sac and tear duct;
• inflammatory phenomena in the conjunctival membrane;
• episcleritis;
• inflammation of the optic nerve.

The nervous system is involved in a pathological process in about a third of cases. Internists discover the following forms of defeat:

1. Neuropathy.
2. Neuritis of the cranial nerves (5 and 7 pairs).
3. Mononeuritis (mostly asymmetric).

The cardiac muscle and the gastrointestinal tract are affected nonspecifically. Myocardial diseases are approximately the same as in the general population.

The presence of functional disorders of the heart and organs of the gastrointestinal tract requires the approach of appropriate specialists.

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Wegener's granulomatosis diagnosis

In order to verify such a serious pathology, an individualized and criterial approach is needed.

It is important to see the ENT doctor. It determines the nature of the inflammation of the nasal mucosa and, if possible, the paranasal sinuses. Usually, a competent specialist does not have problems with the detection of this form of inflammation.

Radiography of the lungs reveals a second important criterion of the disease - the appearance of nodes or infiltrative shadows, which are very prone to decay. It is important to pay attention to the dynamics of these formations, because with Wegener's granulomatosis they are "volatile".

In the diagnosis, it is also important to study the titer of antineutrophil antibodies. Their typical differentiation allows predicting the outcome of the disease and treatment.

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Approaches to therapy

Wegener's granulomatosis is a problem of rheumatologists. Treatment of it requires the use of immunosuppressants and hormonal drugs.

In addition, the multiplicity of lesions dictates the need for symptomatic treatment, taking into account the recommendations of a cardiologist, gastroenterologist, neurologist, dermatologist, nephrologist, pulmonologist.

Drugs that have an immunosuppressive effect are cytostatics. They are appointed courses. Cyclophosphamide is most commonly used.

When treating this drug, monitor the following indicators:

• the level of platelets;
• the content of hepatic transaminases;
• hemoglobin;
• the concentration of creatinine.

An alternative to the use of cyclophosphamide is Metipred. It is a derivative of glucocorticoid hormones, which has a powerful anti-inflammatory and cytotoxic effect. In refractory cases, a combination of drugs is justified.

With terminal kidney damage with the development of chronic renal failure, doctors are forced to recommend hemodialysis. An alternative is peritoneal dialysis. Kidney transplantation is possible.

The prognosis of the disease without treatment measures is disappointing for both doctors and the patient. More than 90% die in the first year after the verification of the disease.

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Timely-initiated therapy with cyclophosphamide results in a significant regression of symptoms and laboratory indicators of the disease in most cases.

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The five-year survival rate increases to 70%.

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A source: http://prososudi.ru/bolezni/vaskulit/lechenie-granulematoz-vegenera.html

Wegener's granulomatosis - symptoms, treatment, forms, stages, diagnosis

Wegener's granulomatosis is a systemic granulomatous-necrotic giant cell vasculitis that initially affects the upper and lower respiratory tract, and in the future - the kidneys.

There are no reliable statistical data on the incidence of Wegener's granulomatosis and its prevalence. The disease is more common in men of young and middle age, almost never occurs in childhood.

Causes and risk factors

The exact causes of Wegener's granulomatosis are unknown. Given that the immune system is found in the blood of the disease under consideration, it is classified as an autoimmune pathology.

Presumably, the disease develops as a result of an error in the immune system, due to which the tissues of the vascular walls are perceived by the immune system as alien and are subject to destruction.

This can lead to various inflammatory processes, including infectious.

Antibodies damage the walls of blood vessels, which leads to the formation of areas of inflammation, that is, granulomas. Gradually increasing in size, granulomas cover the lumen of the vessel.

As a result, blood supply to tissues, processes of tissue respiration and metabolism are disturbed.

Forms of the disease

Depending on the extent of the pathological changes, two forms of Wegener's granulomatosis are distinguished:

• localized. The upper part of the respiratory system is mainly affected, which is manifested by the hoarseness of the voice, frequent nosebleeds, the formation of bloody crusts in the nasal cavity, persistent rhinitis;
• generalized. Characterized by extensive systemic manifestations (fever, abscessed pneumonia, joint-muscle pain, an increase in signs of renal, cardiovascular and respiratory failure).

Stages of the disease

In the clinical picture of Wegener's granulomatosis, several stages are distinguished:

1. Rinogenous granulomatosis (granulomatous-necrotic vasculitis). Developed ulcerative-necrotic or purulent-necrotic rhinosinusitis, nasopharyngitis, laryngitis, destructive changes in the orbit and nasal septum.
2. Pulmonary granulomatosis. The pathological process captures the lung tissue.
3. Generalized granulomatosis. The lower parts of the respiratory system, the cardiovascular system, the organs of the digestive tract and the kidneys are affected.
4. Terminal granulomatosis. Renal and / or pulmonary insufficiency develops. The lethal outcome occurs within a year from the moment of transition of the disease to the terminal stage.

Symptoms of Wegener's granulomatosis

The general symptoms of Wegener's granulomatosis include:

• fever with chills;
• severe weakness;
• hyperhidrosis (increased sweating);
• pain in the joints and muscles;
• loss of body weight.

In 90% of patients, Wegener's granulomatosis manifests itself in the upper respiratory system. Formed chronic rhinitis, resistant to ongoing therapy, which is accompanied by purulent-bloody discharge.

Ulcerative defects are formed on the mucous membrane of the nasal cavity. Gradually increasing in size, they lead to perforation of the nasal septum, saddle-shaped deformation of the nose.

The presence of granulomatous necrotic changes also appears in the paranasal sinuses, external auditory canals, trachea, larynx and oral cavity.

In 2/3 patients, the disease is accompanied by the formation of destructive cavities in the lungs, persistent cough, hemoptysis.

Involvement of renal vessels in the pathological process leads to the development of glomerulonephritis, and in the future - renal failure.

Skin symptoms of Wegener's granulomatosis consist in the appearance of a hemorrhagic rash, the elements of which are prone to necrosis.

Eye, nervous system, myocardium, coronary arteries can also be affected.

Diagnosis of Wegener's granulomatosis

When suspected of Wegener's granulomatosis, an instrumental laboratory examination is performed:

• general blood test (for thrombocytosis, acceleration of ESR, normochromic anemia);
• general urine analysis (microhematuria, proteinuria are detected);
• biochemical analysis of blood (to increase haptoglobin, seromucoid, fibrin, urea, creatinine, γ-globulin);
• Radiography of the lungs (on pleural exudate, decay cavities, infiltrates);
• immunodiagnostics (a decrease in the level of complement, the presence of antineutrophil antibodies, antigens of class HLA - B8, B7, DR2 and DQW7) are established.

If necessary, other methods are used to diagnose Wegener's granulomatosis: for example, computer tomography or tissue biopsy of the upper respiratory tract followed by a histological analysis.

Treatment of Wegener's granulomatosis

The main method of treatment of Wegener's granulomatosis is immunosuppressive therapy, which is performed by a combination of cyclophosphamide and prednisolone.

As the patient's condition improves, the dosage is gradually reduced to a complete cancellation.

After this, the patient is prescribed methotrexate for a long course (-2 years).

In the therapy of the generalized form of Wegener's granulomatosis, extracorporeal hemocorrection methods are used:

• extracorporeal pharmacotherapy;
• cascade filtration of plasma;
• plasmapheresis;
• cryoapheresis.

Possible complications and consequences

Progressing course of Wegener's granulomatosis can be complicated:

• hemoptysis;
• deafness;
• destruction of the bones of the facial skull;
• gangrenous feet;
• the formation of necrosis sites in the lung parenchyma;
• secondary infections on the background of immunosuppressive therapy;
• renal insufficiency.

Prognosis for Wegener's granulomatosis

The prognosis for Wegener's granulomatosis in the absence of adequate and timely treatment is extremely unfavorable. More than 90% of patients die in the first two years from the date of diagnosis.

Against the backdrop of immunosuppressive therapy, 85% of patients showed significant improvement, in 75% of patients the disease passes into the stage of remission. More than half of the cases of remission last about a year, after which there is an exacerbation.

In 13% of patients, the disease is resistant to ongoing immunosuppressive therapy and is characterized by a constantly progressive course.

Prevention

In view of the fact that the exact cause of the occurrence of Wegener's granulomatosis is unknown, measures for its prevention have not been developed.

A source: http://www.neboleem.net/granulematoz-vegenera.php

Full review of Wegener's granulomatosis disease: causes, types, treatment

In our today's article, we will talk about an extremely rare and severe disease like Wegener's granulomatosis. Its diverse clinical manifestations, the difficulties of diagnosis and treatment.

articles:

• Causes
• Forms and symptoms of the disease
• Diagnostics
• Methods of treatment
• Forecast

Wegener's granulomatosis is the most complicated autoimmune disease in which small and medium-sized vessels are affected. Fortunately, the disease is rare.

The average incidence in the world is about 3-4 episodes per 100 000 population, but these figures may be higher due to the difficulties of setting a specific diagnosis.

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Many patients are already posthumously diagnosed with this diagnosis, and during the course of their life the symptoms of the disease are mistaken for very different diseases.

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Representatives of both sexes are equally sick, and for the first time the disease usually appears at the age of 30-50 years. We will try to understand in more detail what is happening with this disease.

Wegener's granulomatosis refers to the so-called autoimmune vasculitis - inflammatory vascular disease. It is believed that inflammation occurs against the background of the invasion of pathogenic viruses or bacteria.

In the case of autoimmune diseases, the body itself becomes the cause of inflammation, cells of the immune system of which, for as yet obscure reasons, "declare a hunt" for their own organs and tissues.

In the case of Wegener's granulomatosis, the walls of small vessels become the target of immune cells: arterioles, venules, capillaries.

The disease is characterized by a sudden onset, rapid and even lightning development and extremely bad consequences. Without proper treatment, the patient dies within 6-12 months. On the background of therapy the patient can live 5-10 years and more.

The treatment of autoimmune diseases, as a rule, is done by a rheumatologist, but on the way to it the patient is faced with a huge number of doctors of related specialties: otorhinolaryngologists, nephrologists, urologists, pulmonologists, phthisiatricians and even oncologists. Symptoms of this pathology are so multifaceted that they seize many organs and simulate a huge number of diseases. About the clinical manifestations and forms of the disease, we'll talk just below.

Causes of pathology

Strictly speaking, the causes of all autoimmune diseases are not known for certain. However, scientists around the world have identified a certain relationship between the aggression of their own immune system to their native cells (these are common causes for all autoimmune diseases):

1. Postponed viral infections. Recently, much attention has been paid to the Epstein-Barr virus or the infectious mononucleosis virus. Also, possible causes of autoimmune reactions are called influenza, herpes, cytomegalovirus.
2. Pronounced allergic reactions, as well as their aggressive treatment.
3. Unreasonable and unnecessary interventions in the immune system, including the use of various drugs "for immunity so popular among patients.
4. Admission of serious medical drugs for the treatment of cancer, tuberculosis, herpetic infections.
5. Hormonal changes in the body, including the transitional age, pregnancy, menopause. Perhaps, that is why autoimmune diseases in general are more often affected by women.

Infectious mononucleosis is one of the possible causes of Wegener's granulomatosis

Forms and symptoms of the disease

It is very important to mention that Wegener's granulomatosis has some favorite localizations, according to which several forms or localizations of the disease have been created:

1. The local form - damage to the organs of hearing, nasal cavity and nose walls - is the most common form. A similar variant develops in 90% of patients.
2. The local form is the defeat of the eyes, namely sclera, iris and conjunctivitis. This form is less common, in about 10% of cases.
3. The local form is cutaneous manifestations of vasculitis. Similar vasculitis is described in 40% of patients.
4. The generalized form - lesion of lung tissue, trachea and bronchi - is one of the typical forms of the disease. The broncho-pulmonary system suffers in 80-85% of patients.
5. The generalized form - renal damage - is observed in 60-70% of patients.
6. The generalized form - the defeat of the nervous system - in about 15% of patients.
7. Combined forms - are found in almost 100% of cases, that is, there will be a combination of two or more forms.

The first symptoms of Wegener's granulomatosis are not very specific.

Patients may complain of general weakness, fatigue, a slight increase in temperature, pain in the muscles and joints, poor appetite and weight loss.

Similar symptoms are typical for any cold or chronic fatigue syndrome, so most patients do not attach much importance to them. Specific manifestations of the disease appear somewhat later.

Most often, the disease begins with an otorhinolaryngological form or eye injury, and after a while - within several months - this or that generalized form develops, which becomes lethal. Let's go through each of the clinical forms of the disease.

Otorhinolaryngological form

With the defeat of ENT organs, the most typical are inflammation and the subsequent necrosis of the walls and septa of the nose. Initial symptoms can proceed according to the type of inflammation of the nose - rhinitis, inflammation of the paranasal sinuses - sinusitis.

1. There are swelling and swelling of the mucous membranes, nasal congestion.
2. Nasal bleeding is associated with ulcers and hemorrhages in the nasal cavity.
3. When the cartilage of the nose and the bony septa of the nose are damaged, these tissues are gradually destroyed, the nose changes its shape - the septum of the nose collapses, the back collapses.

Less often there are ear lesions, manifested by chronic and not responding to conventional otitis media. A prolonged course of the disease leads to the development of deafness.

Ophthalmic form

Eye damage often becomes the first typical sign of Wegener's granulomatosis.

The eye shape is characterized by the appearance of inflammation of the sclera of the eye - scleritis and the choroid of the eye - uveitis.

The disease is characterized by a marked reddening of the eye, eyelid edema, pain and burning sensation. Against the backdrop of these processes, there is a decrease in vision up to complete blindness.

Skin Form

Skin manifestations of autoimmune vasculitis are called purpura. Purple is characterized by the appearance of dense red or crimson spots of various sizes, slightly rising above the surface of the skin. In fact, these spots are the result of hemorrhages from damaged vessels under the skin.

Eruptions are painless, can merge with each other, sometimes - spontaneously disappear. In rare cases, against the background of secondary infection, the sites can be subjected to inflammation and necrosis. Sometimes ulcers appear on the skin.

Such skin lesions need to be distinguished from a huge number of other vasculitides - kA autoimmune, and related to the blood coagulation system and the pathology of the vascular wall.

Purpura is a skin manifestation of pathology

Pulmonary form

The onset of the disease can proceed according to the type of bronchitis or tracheitis, accompanied by a dry, low-productivity cough.

In the lung tissue, those granulomas or nodules are formed, which eventually decompose.

When the bacterial infection is attached, the granulomas are transformed into cavities of purulent decay, sometimes their opening is accompanied by pulmonary hemorrhage.

Complaints of patients are joined by a cough with blood or pus, shortness of breath, chest pain.

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In some patients, such complaints may be absent, so a diagnosis can be assumed based on an X-ray image of the lungs.

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Unfortunately, the primary diagnosis is most often tuberculosis or lung cancer.

Death can come from acute respiratory failure or pulmonary hemorrhage.

Renal form

This is perhaps the most serious and dangerous form of Wegener's syndrome, leading to mortality and severe disability of patients. Kidneys, in fact, are a kind of ball of small vessels surrounded by parenchymal tissue.

It is these vessels that are very often affected by the disease, causing a specific kidney damage - glomerulonephritis.

Glomerulonephritis can be caused by various factors, including streptococcal toxins, poisons, medicinal means, however, in the case of Wegener's syndrome, the disease proceeds with lightning speed, with a rapid onset of renal disease insufficiency.

Most often, patients make the following complaints:

1. I thirst, dry mouth.
2. Swelling and uncaused increase in body weight.
3. Lethargy, drowsiness, apathy.
4. Increased blood pressure and related symptoms: headache, tinnitus, heart pain and so on.
5. Change in the amount of urine.
6. When analyzing urine, a large amount of protein is detected, erythrocytes, a change in the density of urine takes place.

Unfortunately, very often kidney disease is detected already at the stage of development of chronic renal failure, with a lesion heart and other internal organs by the products of the decomposition of one's own organism, which the dying kidneys are unable to excrete with urine.

Neuropathic form

The disease mainly affects the peripheral nerve endings - there is polyneuropathy. This condition is characterized by the following symptoms:

1. A violation of the sensitivity of the skin, especially the area of ​​the limbs.
2. The presence of abnormal "skin" sensations - crawling, numbness of the skin, sensations of heat and cold.
3. Paralysis and weakness of the muscles of the extremities.

This form of the disease is distinguished from many other neuropathies: from banal vitamins B to multiple sclerosis.

Diagnostics

As we have already understood, the diagnosis of Wegener's granulomatosis is an extremely difficult task.

It should be distinguished from malignant tumors, kidney diseases, tuberculosis, sarcoidosis, blood diseases, various complex forms of otitis, sinusitis, uveitis, as well as from a huge number of other autoimmune diseases such as lupus, hemorrhagic vasculitis, syndrome Goodpathra.

For the initial stages of diagnosis, the following studies are needed:

• Clinical tests of blood and urine can indicate the presence of an inflammatory process, anemia, detect traces of protein and blood in the urine, a change in its relative density.
• A biochemical blood test will indicate signs of kidney and liver damage.
• X-ray and computer tomography studies will indicate the presence of granulomas and foci of disintegration in the lungs and bronchi.
• Ultrasound examination of the abdominal organs and kidneys will indicate the damage to the kidneys, a decrease in their blood flow.
• Additional urological tests such as excretory urography, scintigraphy will indicate the degree of decrease in kidney function.
• Consultations of related specialists: ENT doctor, ophthalmologist, dermatologist, urologist, nephrologist, pulmonologist will help to exclude other similar diseases.

Scintigraphy is optional, but one of the additional methods for diagnosing Wegener's granulomatosis. With scintigraphy, a small amount of a radioactive substance is introduced into the body, which then, when emitting radiation, gives an image on a gamma tomograph

The main specific test, which with a high degree of probability will indicate exactly the granulomatosis Wegener is a blood test for the presence of classical antineutrophil cytoplasmic antibodies (CHANCELLA). These are special antibodies that attack cells of the immune system - neutrophils. Such antibodies are detected with several autoimmune vasculitides, including Wegener's disease.

Methods of treatment

Given that the disease is autoimmune, the main group of drugs for the treatment of Wegener's disease are immunosuppressants - drugs to suppress immune defense.

At the moment, two main groups of immunosuppressants are distinguished:

1. Cytotoxic agents are cyclophosphamide, methotrexate, fluorouracil.
2. Glucocorticosteroid hormones - prednisolone, dexamethasone.

These drugs affect the "raging" immunity, suppressing its reaction. Unfortunately, in addition to this link, drugs reduce the general resistance of the body to infections, the growth of skin cells and mucous membranes, disrupt the metabolism.

The earlier the disease is detected and confirmed, the earlier the therapy is started, the better the predictions of the patients. Patients should receive treatment consistently.

Doses and the mode of administration of drugs are selected by a rheumatologist.

Some patients are well suited to the so-called pulse therapy - the introduction of drugs in large doses once or twice a week.

Such groups of patients are categorically contraindicated in any means for stimulating immunity, even plant origin, alcohol, many groups of antibiotics and cardiac drugs.

Prognosis for the disease

In general, with Wegener's granulomatous disease, the prognosis is unfavorable. Without proper treatment, patients die within the first year of the disease. About 90% of patients against the background of timely treatment begun, the average life expectancy is 5 years or more.

The greatest complexity for treatment is made by pulmonary and especially kidney forms, with their overall survival not exceeding 4 years.

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Diagnosis and treatment of Wegener's granulomatosis

Wegener's granulomatosis is an autoimmune disease characterized by necrotic lesions of the arteries. With this disease, there is a defeat of the respiratory and urinary systems. These features affect the mechanism of development of the pathological process.

The main reasons for its occurrence remain unclear. Some patients show definite antibodies in the blood, lung and kidney tissues. The development of granulomas is promoted by failures in the work of the immune system, arising for unknown reasons.

Wegener's granulomatosis is more often found in men 40-75 years old.

The main signs of the disease

In the early stages of the disease symptoms are similar to the manifestations of influenza and ARVI. The patient consults a doctor with signs of rhinitis, maxillary sinusitis, middle ear inflammation and occlusion of the auditory canal.

May worsen hearing and the appearance of pain in the ears. When examined, granulomatous formations are found in the tissues of the nasopharynx, nasal sinuses and soft palate.

With the further development of the pathological process, symptoms such as fever, general weakness, and decreased appetite appear.

In some patients, early signs of the disease show signs of a defeat of the respiratory system - cough, chest pain, sputum with blood, dyspnea.

Dangerous for life massive bleeding occurs rarely. In most cases, violations of the respiratory system appear in the late stages of the disease.

Diagnosis of Wegener's granulomatosis is carried out by X-ray examination.

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In the picture there are clearly visible single and multiple infiltrates, segmental atelectasis, signs of exudative pleurisy. Increases in regional lymph nodes are not observed.

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Sinusitis is often accompanied by purulent infections, which requires the appointment of aggressive antibiotic therapy.

Often the symptoms of nasopharyngeal lesions are mild, only after a while there are signs of systemic pathology.

In medical practice, there were cases in which generalized forms of the disease developed several years after the diagnosis of Wegener's granulomatosis.

The perforation of the hard palate for granulomatous vasculitis is not typical.

The defeat of the excretory system is observed in 90% of cases, the appearance of symptoms of renal failure indicates the generalization of the pathological process.

The general analysis of urine reflects the changes characteristic of acute glomerulonephritis - in the urine there are impurities of blood and protein.

At a biopsy the focal necrosis of kidney tissues is revealed.

In 10% of cases, symptoms of transient glomerulonephritis are detected with the appearance of necrotic changes in the tissues of the kidneys and azotemia.

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Wegener's granulomatosis leads to damage to the organs of vision in about half of the patients. In rare cases, the proliferation of granulomatous tissues contributes to the development of thrombosis of the arteries of the fundus and the subsequent loss of vision.

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With the same frequency, symptoms of skin lesions are detected - spot hemorrhages located on the arms and legs. Papillary rashes, vesicles and necrotic skin lesions are extremely rare.

In most patients, Wegener's granulomatosis leads to inflammation of large joints. Pathological changes are reversible.

Symptoms of joint damage in this disease are often confused with manifestations of rheumatoid arthritis.

Granulomatosis with polyangiitis can lead to impairment of the central nervous system.

Germination of granulomatous formations in the brain leads to the appearance of signs of diabetes insipidus and the defeat of cranial nerves.

Very rarely, Wegener's granulomatosis affects the digestive organs, with characteristic symptoms appearing. Diagnosis of the disease in this case is facilitated by the presence of signs of damage to the respiratory and excretory systems.

Methods of diagnosis

Diagnosis of the disease begins with a general blood test, the results of which reflect hyperglobulinemia, neutrophilic leukocytosis, increased ESR. Analysis for viral hepatitis gives a negative result.

To determine the degree of renal failure, urine tests should be performed and the level of creatinine monitored.

The diagnosis of Wegener's granulomatosis in the early stages is carried out on the basis of the patient's symptoms of the disease.

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Diagnosis of the disease is facilitated by the presence of infiltrates in the lungs, symptoms of kidney failure, and proptosis.

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An important role in the detection of the disease is biopsy of the affected mucous membranes of the nasopharynx and sinuses.

The main histological sign of granulomatous vasculitis is the presence of areas of necrosis and granulomatous formations. With true vasculitis, such changes are not observed.

Similar to granulomatous vasculitis, a clinical picture may have some oncological diseases, for example, lymphoma.

The same applies to lymphomatoid granulomatosis, which is considered a precancerous condition.

Wegener's granulomatosis is more difficult to distinguish from the median granuloma.

This rare disease is characterized by the rapid development of the inflammatory process, leading to destruction of the facial tissues and upper respiratory tract. The causes of this pathology remain unclear.

The first symptoms of the median granuloma are permanent nasal congestion, the appearance of purulent discharge, ulceration of the mucous membranes of the nasopharynx.

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In the late stages of the disease there is a lesion of the sinuses, a significant increase in temperature, tooth loss, destruction of the nasal septum, hard and soft palate.

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There is a widespread necrosis of the tissues of the nasopharynx and the face, accompanied by the appearance of a sharp unpleasant odor. Untimely or incorrect treatment leads to the development of severe complications that cause death.

The process does not affect the respiratory and excretory systems, which differs from Wegener's granulomatosis.

Treatment of vasculitis

If the disease is not detected in time, the prognosis is always unfavorable. More than 90% of patients die within 2 years after the appearance of the first symptoms of the disease.

When generalizing the pathological process, the patient's life expectancy is reduced to 6 months. Treatment with corticosteroids, as a rule, is not effective enough. Therefore, they are replaced with Cyclophosphamide taken internally.

With the rapid development of the disease treatment is carried out by intravenous administration of the drug.

Treatment begins with the introduction of increased doses of prednisolone, with the improvement of their condition gradually reduce. Cyclophosphamide is used for long courses.

Gradual reduction of its dose begins only a year after entering the stage of remission.

Proper treatment contributes to the disappearance of signs of the disease in 95% of cases.

The period of remission can last more than 20 years. Relapses occur extremely rarely, treatment in this case ends in recovery.

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There is information about the high efficacy of Methotrexate, Azathioprine and Chlorbutin. However, these drugs are used very rarely, so treatment is most often performed with Cyclophosphamide.

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Since the causes of the disease are not clear, its prevention is impossible.

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